Objective The objective of this study was to review the long-term results of symptomatic patients with Ebstein anomaly in the neonatal period. Methods The medical records of 40 neonates with a diagnosis of Ebstein anomaly who were admitted to our institution between January 1988 and June 2008 were retrospectively reviewed. Primary outcomes studied included patient survival and need for reintervention. Results No early intervention was required in 16 of the 40 patients with a hospital survival of 94% (15/16) and no late mortality. The remaining 24 patients underwent surgical intervention in the neonatal period. A shunt alone was performed in 9 patients with an actuarial survival of 88.9% at 1 year and 76.2% at 5 and 10 years. For the patients undergoing intervention on the tricuspid valve, survival estimates for the 11 patients with a right ventricular exclusion procedure were 63.6% at 1, 5, and 10 years and 47.7% at 15 years compared with 25.0% at 1, 5, and 10 years for the 4 patients with tricuspid valve repair. All long-term survivors were in New York Heart Association class I or II, and only 1 patient required antiarrhythmic medication. Conclusion Symptomatic neonates with Ebstein anomaly requiring no intervention or shunting alone have good long-term survival. For patients needing intervention on the tricuspid valve, overall survival is lower. For these patients, right ventricular exclusion may be superior to tricuspid valve repair.